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Rabbit Anti-URP2/Kindlin-3  antibody (bs-13738R)  
~~~促銷代碼KT202411~~~
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產品編號 bs-13738R
英文名稱 Rabbit Anti-URP2/Kindlin-3  antibody
中文名稱 整合素結合性信號分子抗體
別    名 Fermitin family homolog 3; Fermitin family member 3; FERMT3; Kind3; Kindlin 3; Kindlin-3; Kindlin3; MGC10966; MIG 2; MIG2 like protein; MIG2-like protein; MIG2B; Unc 112 related protein 2; Unc-112-related protein 2; UNC112C; URP2_HUMAN; URP2SF.  
研究領域 腫瘤  細胞生物  信號轉導  細胞粘附分子  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human,Mouse,Rat,Pig,Cow,Horse)
產品應用 IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 76kDa
細胞定位 細胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human URP2: 401-500/667 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 Kindlin-3 is a 667 amino acid protein that localizes to both the cell membrane and the cytoplasm and contains one PH domain and one FERM domain. Expressed at high levels in lymph node tissue and at lower levels in spleen, thymus, stomach, placenta, lung, testis and small intestine, Kindlin-3 is thought to be involved in cell adhesion events and may play a role in apoptosis. Kindlin-3 is overexpressed in B-cell malignancies, suggesting that, via its ability to affect cell adhesion, Kindlin-3 may participate in tumor transformation and metastasis. Two isforms of Kindlin-3, designated short and long, exist due to alternative splicing events.

Function:
Plays a central role in cell adhesion in hematopoietic cells. Acts by activating the integrin beta-1-3 (ITGB1, ITGB2 and ITGB3). Required for integrin-mediated platelet adhesion and leukocyte adhesion to endothelial cells. Required for activation of integrin beta-2 (ITGB2) in polymorphonuclear granulocytes (PMNs).
Isoform 2 may act as a repressor of NF-kappa-B and apoptosis.

Subcellular Location:
Cell projection; podosome. Present in the F-actin surrounding ring structure of podosomes, which are specialized adhesion structures of hematopoietic cells.

Tissue Specificity:
Highly expressed in lymph node. Expressed in thymus, spleen and leukocytes. Weakly expressed in placenta, small intestine, stomach, testis and lung. Overexpressed in B-cell malignancies.

DISEASE:
Defects in FERMT3 are the cause of leukocyte adhesion deficiency type 3 (LAD3) [MIM:612840]; also called leukocyte adhesion deficiency 1 variant (LAD1v). LAD3 is a rare syndrome characterized by infections without pus formation in the presence of a leukocytosis combined with a Glanzmann-type bleeding disorder, resulting from a hematopoietic defect in integrin activation. Symptoms arise from an inability to activate the integrins expressed on hematopoietic cells, including platelets and leukocytes.

Similarity:
Belongs to the kindlin family.
Contains 1 FERM domain.
Contains 1 PH domain.

SWISS:
Q86UX7

Gene ID:
83706

Database links:

Entrez Gene: 83706 Human

Entrez Gene: 525159 Cow

Entrez Gene: 108101 Mouse

Entrez Gene: 309186 Rat

Omim: 607901 Human

SwissProt: Q32LP0 Cow

SwissProt: Q86UX7 Human

SwissProt: Q8K1B8 Mouse



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